Defining the mechanism of polymerization in the serpinopathies (Q34182669)

31 July 2017

title

Defining the mechanism of polymerization in the serpinopathies (English)

1 reference

31 July 2017

Elena Miranda

3

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Bibek Gooptu

object named as

Bibek Gooptu

4

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Matthew F. Bush

object named as

Matthew F Bush

5

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Carol V. Robinson

object named as

Carol V Robinson

8

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author name string

Ugo I Ekeowa

1

1 reference

31 July 2017

Joanna Freeke

2

1 reference

31 July 2017

Juan Pérez

6

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31 July 2017

Jeff Teckman

7

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31 July 2017

David A Lomas

9

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31 July 2017

language of work or name

English

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publication date

20 September 2010

1 reference

Proceedings of the National Academy of Sciences of the United States of America

31 July 2017

published in

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31 July 2017

volume

107

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31 July 2017

issue

40

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31 July 2017

page(s)

17146-17151

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A novel monoclonal antibody to characterize pathogenic polymers in liver disease associated with alpha1-antitrypsin deficiency.

31 July 2017

cites work

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2951428

Subunit architecture of multiprotein assemblies determined using restraints from gas-phase measurements

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2951428

Neuroserpin polymers activate NF-kappaB by a calcium signaling pathway that is independent of the unfolded protein response.

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2951428

Small-molecule peptides inhibit Z alpha1-antitrypsin polymerization

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2951428

Crystal structure of a stable dimer reveals the molecular basis of serpin polymerization

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2951428

The structural basis of serpin polymerization studied by hydrogen/deuterium exchange and mass spectrometry

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2951428

Conformation of macromolecules in the gas phase: use of matrix-assisted laser desorption methods in ion chromatography.

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2951428

Accumulation of mutant alpha1-antitrypsin Z in the endoplasmic reticulum activates caspases-4 and -12, NFkappaB, and BAP31 but not the unfolded protein response.

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2951428

Novel serpinopathy in rat kidney and pancreas induced by overexpression of megsin

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2951428

Quantitative isolation of alphalAT mutant Z protein polymers from human and mouse livers and the effect of heat

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2951428

Peptides and proteins in the vapor phase

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2951428

Cleaved antitrypsin polymers at atomic resolution

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2951428

Inactive conformation of the serpin alpha(1)-antichymotrypsin indicates two-stage insertion of the reactive loop: implications for inhibitory function and conformational disease

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2951428

A 2.6 A structure of a serpin polymer and implications for conformational disease

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2951428

Structural transition of alpha 1-antitrypsin by a peptide sequentially similar to beta-strand s4A.

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2951428

Accumulation of the insoluble PiZ variant of human alpha 1-antitrypsin within the hepatic endoplasmic reticulum does not elevate the steady-state level of grp78/BiP.

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2951428

The mechanism of Z alpha 1-antitrypsin accumulation in the liver

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2951428

Conformational pathology of the serpins: themes, variations, and therapeutic strategies

28 July 2018

1 reference

https://api.crossref.org/works/10.1073/PNAS.1004785107

The mechanism of alpha 1-antitrypsin polymerization probed by fluorescence spectroscopy

21 January 2018

1 reference

https://api.crossref.org/works/10.1073/PNAS.1004785107

6-mer peptide selectively anneals to a pathogenic serpin conformation and blocks polymerization. Implications for the prevention of Z alpha(1)-antitrypsin-related cirrhosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1073/PNAS.1004785107

Alpha 1-antitrypsin polymerization: a fluorescence correlation spectroscopic study.

21 January 2018

1 reference

https://api.crossref.org/works/10.1073/PNAS.1004785107

How small peptides block and reverse serpin polymerisation.

21 January 2018

1 reference

https://api.crossref.org/works/10.1073/PNAS.1004785107

Topography of a 2.0 Å structure of α1-antitrypsin reveals targets for rational drug design to prevent conformational disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1073/PNAS.1004785107

Probing hemoglobin structure by means of traveling-wave ion mobility mass spectrometry

21 January 2018

1 reference

https://api.crossref.org/works/10.1073/PNAS.1004785107

Deciphering drift time measurements from travelling wave ion mobility spectrometry-mass spectrometry studies

21 January 2018

1 reference

https://api.crossref.org/works/10.1073/PNAS.1004785107

New Findings in PiZZ alpha1-antitrypsin deficiency-related panniculitis. Demonstration of skin polymers and high dosing requirements of intravenous augmentation therapy

21 January 2018

1 reference

https://api.crossref.org/works/10.1073/PNAS.1004785107

Probing neuroserpin polymerization and interaction with amyloid-beta peptides using single molecule fluorescence.

21 January 2018

1 reference

https://api.crossref.org/works/10.1073/PNAS.1004785107

A Kinetic Mechanism for the Polymerization of α1-Antitrypsin

21 January 2018

1 reference

https://api.crossref.org/works/10.1073/PNAS.1004785107

Ion mobility–mass spectrometry analysis of large protein complexes

21 January 2018

1 reference

12 December 2020

Effect of the Z mutation on the physical and inhibitory properties of alpha 1-antitrypsin

1 reference

12 December 2020

The natural history of liver disease in alpha 1-antitrypsin deficient children

1 reference

12 December 2020

Determining the stoichiometry and interactions of macromolecular assemblies from mass spectrometry

1 reference

12 December 2020

Cooperative unfolding of a metastable serpin to a molten globule suggests a link between functional and folding energy landscapes

1 reference

12 December 2020

Identifiers

DOI

10.1073/PNAS.1004785107

1 reference

31 July 2017

0 references

1 reference

31 July 2017

1 reference